Novel treatment strategies in congenital adrenal hyperplasia

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Congenital Adrenal Hyperplasia and Schmid Metaphyseal Chondrodysplasia in a Child

Congenital adrenal hyperplasia (CAH) is a group of hereditary diseases, which are autosomal recessive. CAH occurs due to defect in one of the cortisol coding genes and often clinically presents itself with signs of androgen overproduction. In this article, we report a case of CAH and Schmid metaphyseal dysplasia. Our literature review indicated that this report is the first attempt on CYP11B1 a...

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Monitoring treatment in congenital adrenal hyperplasia.

We report results of monitoring treatment in 41 patients with congenital adrenal hyperplasia controlled over 0.3-13.1 years using standard auxological techniques alone. Doses of glucocorticoid (15-25 mg/m2/day) and mineralocorticoid (0.15 mg/m2/day) replacement were determined initially using biochemical indices and thereafter adjusted according to surface area. Monitoring was solely directed a...

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Dexamethasone treatment for congenital adrenal hyperplasia.

Ten patients with congenital adrenal hyperplasia (three males, seven females; aged 12-29 years) had their usual glucocorticoid treatment changed to dexamethasone in three crossover dosage regimens. A starting dose of 5 micrograms/kg/day is suggested but as no one dose regimen resulted in adequate control the timing of the dose must be decided for each patient.

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Classic congenital adrenal hyperplasia.

Congenital adrenal hyperplasia is attributed to inherited enzyme defects in the adrenal cortex. The classical form results in reduced production of cortisol and aldosterone, accompanied by an increase in production of adrenal cortical androgens. This causes virilisation in girls, adrenocortical failure and early puberty in both sexes. This article describes the genetics, clinical picture, diagn...

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Congenital adrenal hyperplasia

Congenital adrenal hyperplasia consists of a heterogenous group of inherited disorders due to enzymatic defects in the biosynthetic pathway of cortisol and/or aldosterone. This results in glucocorticoid deficiency, mineralocorticoid deficiency, and androgen excess. 95% of CAH cases are due to 21-hydroxylase deficiency. Clinical forms range from the severe, classical CAH associated with complete...

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ژورنال

عنوان ژورنال: Current Opinion in Endocrinology & Diabetes and Obesity

سال: 2016

ISSN: 1752-296X

DOI: 10.1097/med.0000000000000256